scholarly journals Patients with Fontan circulation beyond the fourth decade of life

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Gribaudo ◽  
A Constantine ◽  
G Costola ◽  
A Kempny ◽  
M Gatzoulis ◽  
...  
Keyword(s):  
Serum Albumin ◽  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Atrial Arrhythmia ◽  
Morbidity And Mortality ◽  
Lower Serum ◽  
Fourth Decade ◽  
History Of

Abstract Background The survival rate after Fontan procedure is improving and many of these patients now survive well into adulthood. Data on the late morbidity and mortality of Fontan patients in their fourth decade of life are lacking. Purpose To evaluate the late outcome of adult patients with Fontan circulation who have survived beyond 35 years. Methods Data were collected retrospectively on consecutive patients with a Fontan circulation ≥35 years between 2005 and 2019. Baseline (at 35th birthday) and follow-up data includes functional class, blood tests, history of arrhythmia, heart failure (HF), thromboembolism, Fontan related liver disease (FLD), protein losing enteropathy (PLE) and plastic bronchitis. Echocardiographic data on systemic atrioventricular valve (SAVV) regurgitation and ventricular function were also analysed. Results 61 patients were included (29, 47.5% female). Tricuspid atresia was the most common underline anatomy (29,47.5%) and the median age at Fontan procedure was 12 [8.0–19.0] years. 34 (56%) patients had an atrio-pulmonary connection Fontan and 15 (24.5%) total cavo-pulmonary connection (86.7% lateral tunnel and 13.3% extra-cardiac conduit), 10 (16%) a Bjork Fontan and 2 (3%) a Kawashima procedure. At baseline 40 (66%) patients were in NYHA class II or III, 7 (11%) had a moderate or severely impaired ventricular function and 12 (20%) had at least moderate SSAV regurgitation. At baseline, 35 (57.4%) patients had experienced at least one arrhythmia, 7 (11.4%) patients had a history of atrial thrombosis or thromboembolic events and 2 (3.3%) of PLE. At a median follow-up of 6.4 [2.9–11.3] years, 11 (18%) patients died, 7 of chronic HF, 1 hepatic carcinoma, 1 sepsis, 1 pulmonary embolism, 1 malignancy (1 cause unknown). During follow-up, 28 (46%) patients had a new episode of atrial arrhythmia, 23 (38%) developed FLD and 2 (3.3%) PLE. 9 (15%) required at least one admission for HF. 2 (3.3%) patients experienced worsening of their ventricular function and 7 (11.4%) of their SAVV regurgitation. On univariable analysis, a baseline diagnosis of PLE (HR 15.23, 95% CI:2.77–83.86, p=0.002), HF (HR 6.18, 95% CI:1.74–21.99, p=0.005), atrial arrhythmia (HR 4.99, 95% CI:1.07–23.21, p=0.04), lower serum albumin (HR 1.14, 95% CI:1.06–1.23, p=0.0006), lower iron levels (HR 1.31, 95% CI:1.02–1.68, p=0.04) and a higher urea (HR 1.18, 95% CI:1.04–1.34, p=0.01) were associated with death or heart transplantation. On bivariable analysis, lower serum albumin was stronger than all other univariable predictors. Conclusions Adult Fontan survivors are a high risk population with significant morbidity and mortality driven primarily by HF and other long-term complications of the Fontan operation. The predictors of outcome in this cohort reflects the chronic low cardiac output state and systemic venous hypertension. Vigilance is required to identify patients who may benefit of haemodynamic optimization and close monitoring for Fontan-related complications. Funding Acknowledgement Type of funding source: None

Long term follow-up after pregnancy in Fontan patients

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
E Gribaudo ◽  
A Constantine ◽  
A Pires ◽  
I Ahmed ◽  
R Patel ◽  
...  
Keyword(s):  
Ventricular Function ◽  
Nyha Class ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Atrial Arrhythmia ◽  
Class I ◽  
Protein Losing Enteropathy ◽  
Fontan Patients

Abstract Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

Abstract 16329: Survival Prospects in Congenitally Corrected Transposition of the Great Arteries: Results From a Multi-institutional Study

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Alexandra van Dissel ◽  
Alexander Opotowsky ◽  
Jamil A Aboulhosn ◽  
Martijn Kauling ◽  
Salil Ginde ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Transplant ◽  
Cumulative Incidence ◽  
Primary Outcome ◽  
Large Cohort ◽  
Atrial Arrhythmia ◽  
History Of ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Background: Occasionally patients with congenitally corrected transposition of the great arteries (ccTGA) exhibit little clinical evidence of cardiovascular limitation even to their 8th decade. We aimed to assess survival prospects in a large cohort of ccTGA adults. Methods & Results: We included 555 ccTGA adults (median age 33.0 years, 48.3% female) under regular follow-up at 28 institutions between 2002 and 2019. The primary outcome was a composite of death, mechanical circulatory support (MCS) and heart transplant. During a median follow-up of 8.1 [IQR 4.4 - 13.3] years, 56 (10.1%) patients died, 10 (1.8%) patients underwent MCS and 14 (2.5%) had a heart transplant. Median age at time of primary outcome was 51.1 [IQR 37.5 - 63.2] years and cumulative incidence at 15 years from baseline was 21.5% [95% CI 16.1 - 26.5]. Leading causes of death were worsening of heart failure (43%) and sudden death (10%). Patients who died were more likely to use heart failure (HF) medications. In multivariable Cox analyses for baseline variables, age, prior atrial arrhythmia and HF admission were each associated with an increased risk of the primary outcome. Figure shows cumulative incidence according to history of atrial arrhythmia. During follow-up, 91 (16.4%) were admitted for HF, pacemaker implantation was performed in 68 (12.3%) patients, ICD in 82 (14.7%), and major cardiac surgery (mostly for systemic AV-valve) in 89 (15.8%) patients. Conclusion: In this large cohort of ccTGA adults, survival seemed to be primarily determined by heart failure-related complications. Prior atrial arrhythmia also seems to be a harbinger for adverse outcome. Few patients underwent advanced HF therapies. Figure: Cumulative incidence of the composite primary outcome (MCS, heart transplant or death) over a period of 14 years from first visit at an adult congenital heart disease clinic since 2002 stratified according to history of atrial arrhythmia. Shading represents upper and lower 95% confidence limits.

scholarly journals Impact of intrapulmonary-artery septation to pulmonary vein obstruction for two-lung Fontan

2020 ◽  
Vol 58 (1) ◽  
pp. 177-185
Author(s):  
Motonori Ishidou ◽  
Keisuke Ota ◽  
Kentaro Watanebe ◽  
Hiroshi Koshiyama ◽  
Kazuyoshi Kanno ◽  
...  
Keyword(s):  
Pulmonary Circulation ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Pulmonary Venous Obstruction ◽  
Operative Outcomes ◽  
Group 12 ◽  
Lung Area ◽  
Pulmonary Vein Obstruction ◽  
Significant Factors

Abstract OBJECTIVES Patients with unbalanced pulmonary artery (PA) growth and decreased unilateral pulmonary circulation are considered unsuitable candidates for the Fontan procedure. Following our previous study on the utility of intrapulmonary-artery septation for patients with PA hypoplasia, we investigated its use in patients with pulmonary venous obstruction (PVO). METHODS We recruited 42 patients who underwent intrapulmonary-artery septation for unilateral PA hypoplasia and/or PVO between 1998 and 2018 and classified them into no PVO or PVO group. We analysed overall survival, success of the Fontan procedure and data from catheterization and echocardiography. In PVO, we evaluated the functional lung area before the Fontan procedure and the relevance of this parameter to operative outcomes. RESULTS The PVO and no-PVO group included 24 and 18 patients, respectively. One patient in the no-PVO and 6 patients in the PVO group died during follow-up; this difference was statistically significant (log-rank P = 0.040). In the no-PVO group, 15 (83%) patients achieved two-lung Fontan circulation. In the PVO group, 12 (50%) patients achieved two-lung Fontan circulation. Multivariate analysis revealed that functional lung area and shunt size [significantly larger in patients with functional lung area ≥50% in affected lung (P = 0.040)] were significant factors for successful two-lung Fontan procedure (P = 0.030). CONCLUSIONS Intrapulmonary-artery septation may contribute to increase functional lung area after PVO release to establish two-lung Fontan circulation in patients with PVO who have unbalanced PA growth and/or decreased unilateral pulmonary circulation.

scholarly journals Predictors of a Rapid Decline of Renal Function in Patients with Chronic Kidney Disease Referred to a Nephrology Outpatient Clinic: A Longitudinal Study

2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Ana Vigil ◽  
Emilia Condés ◽  
Rosa Camacho ◽  
Gabriela Cobo ◽  
Paloma Gallar ◽  
...  
Keyword(s):  
Cardiovascular Disease ◽  
Chronic Kidney Disease ◽  
Renal Failure ◽  
Renal Function ◽  
Serum Albumin ◽  
Rapid Decline ◽  
Lower Serum ◽  
The Mean ◽  
Kidney Function Decline

Background. Predicting the progression of kidney failure in patients with chronic kidney disease is difficult. The aim of this study was to assess the predictors of rapid kidney decline in a cohort of patients referred to a single outpatient nephrology clinic. Design. Longitudinal, prospective cohort study with a median follow-up of 3.39 years. Methods. Data were obtained from 306 patients with chronic renal failure based on serum creatinine-estimated glomerular filtration rate (eGFRcreat) < 90 mL/min/1.73 m2. After excluding patients who died (n=30) and those who developed end-stage renal failure (n=6), 270 patients were included. This population was grouped according to the rate of kidney function decline. Rapid kidney function decline was defined as an annual eGFRcreat loss > 4 mL/min/1.73 m2. We recorded nonfatal cardiovascular events at baseline and during follow-up in addition to biochemical parameters. Results. The mean loss in renal function was 1.22 mL/min/1.73 m2 per year. The mean age was 75 ± 8.8 years old, and the mean baseline eGFRcreat was 42 ± 14 mL/min/1.73 m2. Almost one-fourth of the sample (23.3% [63 patients]) suffered a rapid decline in renal function. In a logistic regression model with rapid decline as the outcome, baseline characteristics, lower serum albumin (OR: 0.313, 95% CI: 0.114–0.859), previous cardiovascular disease (OR: 1.903 95% CI: 1.028–3.523), and higher proteinuria (g/24 h) (OR: 1.817 CI 95%: 1.213–2.723) were the main predictors of rapid kidney decline. On multivariate analysis, including baseline and follow-up data, we obtained similar adjusted associations of rapid kidney decline with baseline serum albumin and proteinuria. The follow-up time was also shorter in the group with rapid rates of decline in renal function. Conclusion. Renal function remained stable in the majority of our population. Previous cardiovascular disease and cardiovascular incidents, lower serum albumin, and higher proteinuria at baseline were the main predictors of rapid kidney decline in our population.

scholarly journals Fontan circulation and implications for future reproduction

2016 ◽  
Vol 10 (3) ◽  
pp. 104-106
Author(s):  
Niharika Mehta ◽  
Srilakshmi Mitta
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Circulation ◽  
Cardiac Complications ◽  
Preconception Counseling ◽  
Passive Flow ◽  
History Of ◽  
Future Reproduction ◽  
Long Term Prognosis

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

Long-term outcome after atrioventricular valve surgery following modified Fontan operation

2010 ◽  
Vol 21 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Shaji C. Menon ◽  
Joseph A. Dearani ◽  
Frank Cetta
Keyword(s):  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Valve Surgery ◽  
Atrioventricular Valve ◽  
Term Outcome ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome

AbstractObjectiveThe objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.BackgroundAtrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.MethodsPatients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.ResultsA total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.ConclusionAtrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

scholarly journals 2453

2017 ◽  
Vol 1 (S1) ◽  
pp. 29-29
Author(s):  
Angela Weingarten ◽  
Daniel E. Clark ◽  
Ryan D. Byrne ◽  
Patricia Y. Chu ◽  
Frank A. Fish ◽  
...  
Keyword(s):  
Fontan Procedure ◽  
Adult Survivors ◽  
Atrial Arrhythmia ◽  
Morbidity And Mortality ◽  
Right Atrial ◽  
Significant Morbidity ◽  
Heart Transplants ◽  
Systemic Right Ventricle ◽  
Lateral Tunnel ◽  
Significant Difference

OBJECTIVES/SPECIFIC AIMS: The morbidity and mortality in adults with single ventricular hearts who have undergone Fontan palliation is poorly defined. These patients have a high burden of arrhythmia, heart failure, and re-operation. We hypothesized that age and type of Fontan predict occurrence of arrhythmia. METHODS/STUDY POPULATION: In total, 205 patients aged 18 years who had undergone a Fontan procedure were identified. Those with incomplete data were excluded. Demographic, anatomic, pharmacologic, imaging, hemodynamic, and electrophysiologic data were collected. The χ2 and Mann-Whitney U tests were used to test significance defined as p<0.05. RESULTS/ANTICIPATED RESULTS: Of the 205 patients identified, 59 had been lost to follow-up. Of the 146 patients (77, 53% female) actively followed 18 (12%) had died at a median (IQR) age of 27 (21–34.3); in patients alive as of 10/2016 the median age was 26 years (22–34). Fontan types were lateral tunnel (LT) (n=79, 54.1%), extracardiac (EC) (n=32, 22%), right atrial to pulmonary artery (RV-PA) (n=28, 19%), and Fontan with Bjork modification (n=4, 2.7%). Systemic left ventricle (n=96, 66%) was more common than systemic right ventricle (n=43, 30%). Of the 146 patients, 101 (69%) had significant morbidity or mortality: 86 (59%) were diagnosed with arrhythmia, 18 (12%) died, and 11 (8%) underwent heart transplants. Frequent procedures included: Fontan revisions/cryoablation in 28 (19%), electrophysiology studies with ablation in 73 (50%), and pacemakers in 53 (36%). Of the arrhythmia diagnoses, 57 (64%) were atrial tachyarrhythmias. RV-PA Fontan procedures were associated with significantly more atrial arrhythmia than all other Fontan types (70% vs. 30%; p<0.01). There was no statistical difference in occurrence of atrial arrhythmia in adults with LT Versus EC Fontans (p=0.3). While patients who had undergone RV-PA and Bjork Fontans were older with median age 34 years, there was no significant difference in age between LT and EC (median 24.0 and 24.5). DISCUSSION/SIGNIFICANCE OF IMPACT: Adult survivors of the Fontan procedure suffer from significant morbidity and mortality. The single most prevalent morbidity is atrial arrhythmia. We conclude that RV-PA Fontans, now obsolete, have the highest prevalence of arrhythmia and that there is no difference in arrhythmia burden between LT and EC Fontans. Given the high prevalence of morbidity and mortality in this population, it is imperative that they be followed by cardiologists with expertise in congenital heart disease.

scholarly journals Cardiac Myxoma- Surgical treatment and outcome

2015 ◽  
Vol 11 (2) ◽  
pp. 27-30
Author(s):  
D Jaypal Reddy ◽  
Bijay Sah
Keyword(s):  
Malignant Tumors ◽  
Nyha Class ◽  
Cardiac Myxoma ◽  
Malignant Neoplasms ◽  
Class Iii ◽  
Complete Excision ◽  
Fourth Decade ◽  
History Of ◽  
Cardiac Myxomas

Background & Objectives: Cardiac Myxomas include benign and malignant neoplasms, arising within the cardiac chambers or in the myocardium. Because, it is a rare tumor of the heart, there seems a lack of adequate data and its recent optimal treatment for both benign and malignant tumors is limited.Materials & Methods: Literature was searched for review and prospective study of cardiac myxoma surgery done between August 1990 and February 1996 was presented. A total of six patients underwent surgery for cardiac myxoma. All patients (five female; one male) underwent complete excision of the tumor and none of them showed features of remission after six month follow-up.Results: Cardiac Myxoma is a rare disease and it most commonly occurs in the fourth decade of life. Out of six patients operated, five were female and one was male and the age of the patients was between 22 and 50 years (mean age 31). The location of tumors was: LA myxoma in five cases, RA myxoma in one case. Preoperative symptoms were dyspnea (NYHA class III) in four cases, orthopnea in two cases, postural giddiness or syncope in three cases, palpitation in two cases and chest pain in two cases. The mean duration of onset of symptoms to diagnosis was eight months. Echocardiography showed abnormalities in all six cases including mitral stenosis in five cases, and tricuspid stenosis in one case. No recurrence was noted after six months of follow-up after the surgery.Conclusion: Cardiac myxoma is considered an emergency condition which needs urgent procedure, particularly if the patient has a history of embolism or syncope. Atrial Myxoma tends to have more secondary scenarios by embolization.JCMS Nepal. 2015; 11(2):27-30

Designing therapeutic strategies for patients with a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs

2004 ◽  
Vol 14 (6) ◽  
pp. 630-653 ◽  
Author(s):  
Robert M. Freedom ◽  
Shi-Joon Yoo ◽  
Jennifer Russell ◽  
Donald Perrin ◽  
William G. Williams
Keyword(s):  
Left Ventricle ◽  
Myocardial Hypertrophy ◽  
Fontan Procedure ◽  
Therapeutic Strategies ◽  
Fontan Circulation ◽  
Surgical Strategies ◽  
Beneficial Effects ◽  
Early Success ◽  
History Of ◽  
Cavopulmonary Shunt

The palliation of the cyanotic child with a dominant morphologically left ventricle, discordant ventriculo-arterial connections, and obstruction to the pulmonary outflow tract has continued to evolve and mature. The evolution began in the early days of surgical palliation with the Blalock–Taussig shunt, extended to construction of cavopulmonary shunts, if required, and then to the Fontan procedure and its subsequent modifications. This journey took nearly 30 years to complete. There is increasing clinical data to document the beneficial effects of this approach, with ever-improving outcomes. Some aspects of the history of the cavopulmonary shunt have been previously reviewed in this journal and elsewhere,1–5as have analysis of outcomes for some groups of patients considered for surgical completion of the Fontan circulation.6–12While there has been some ongoing interest in ventricular septation since the early success of Sakakibara et al.,13this approach has largely been abandoned. Considerably more challenges and debate resonate in the surgical algorithms defined for patients whose hearts are characterized by a dominant left ventricle, discordant ventriculo-arterial connections, and unobstructed flow of blood to the lungs. This latter group will be the focus of this review, as will the aetiology of the myocardial hypertrophy that is particularly frequent in this group of patients, its clinical recognition, indeed its anticipation, and the multiple surgical strategies designed to prevent or treat it. All these manoeuvres are considered to optimise suitability for, and outcome from, creation of the Fontan circulation.

P0270SKELETAL MUSCLE AREA AT L3 LEVEL INDEPENDENTLY PREDICTS MORTALITY IN ELDERLY PATIENTS WITH ANCA ASSOCIATED VASCULITIS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Xixi Zheng ◽  
Peng Xia ◽  
Jiaying Li ◽  
Gu-mu-yang Zhang ◽  
Yan Qin ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Elderly Patients ◽  
Serum Albumin ◽  
Ct Scan ◽  
Immunoglobulin G ◽  
Muscle Area ◽  
Anca Associated Vasculitis ◽  
History Of ◽  
Skeletal Muscle Area

Abstract Background and Aims Infection is the leading cause of death in elderly patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Older patients are more frail and have poorer nutritional reserves. The decrease in skeletal muscle mass and the development of sarcopenia is a pervasive problem among the elderly. Computer tomography (CT) scan provides a practical method to measure muscle mass in hospitalized patients which has been shown to predict the mortality in cancer and ICU patients. In the present study, we aim in to investigate the relationship between skeletal muscle area and clinical outcomes in elderly AAV patients with renal involvement. Method Hospitalized AAV patients older than 65 years at the diagnosis in Peking Union Medical Hospital from June, 2014 to June, 2019 were enrolled. Skeletal muscle area was calculated on CT image at the third lumbar vertebra level with previously published method using NIH ImageJ software. Baseline Birmingham Vasculitis Activity Score (BVAS), lab results, intensity of immunosuppressive treatment, follow up time and status of the endpoints (infection or death) were recorded. Characteristics between patients with or without endpoint event were compared. Univariate and multivariate Cox regression model was used to determine the independent predictors of mortality. Results A total of 58 patients were included, with. 48.3% male (n=28) and mean age 71.11(65.13, 78.13) years. Baseline BVAS score was 17±4.36 and eGFR was 18.67 (8.306, 34.59) ml/min×1.73m2. Muscle area was 119.6±24.14 cm2 measured on the first CT scan after admission. The baseline serum albumin was 34 (29, 37) g/L, baseline Hemoglobin was 97.83±21.81g/L and baseline immunoglobulin G level was 14.18±4.83g/L. Treatment include glucocorticoid (100%), cyclophosphamide (87.9%), pulse glucocoritcoid (36.2%) and plasma exchange(25.9%). After a median follow-up of 233.5(56.75, 435.8) days, 30 patients experienced 36 episodes of infections that required either hospitalization or administration of antimicrobial agents, of which 7 episodes were complex infections of more than one site and 10 were infections resulting in ICU admission. Median time to infection was 16 (7.5, 59) days from admission. Pneumonia (n=30) and CMV viremia (n=7) were most common. Seven patients died of infection and 1 died of gastrointestinal bleeding. Median time to death was 44 (25.5,75.3) days from admission. In univariate analysis, only age (HR=1.198, CI 1.046,1.372, p=0.006) and muscle area (HR=0.945, CI 0.907,0.986, p=0.009) significantly predicted death. Other variables including BVAS score, eGFR at the onset of disease, history of diabetes, history of pulmonary disease, disease affecting the respiratory system and whether receiving glucocorticoid pulse therapy and baseline nutritional markers i.e. BMI, serum albumin level, lymphocyte count, hemoglobin level and immunoglobulin G level were not significant predictors of death or infection. In multivariate survival analysis, muscle area were significant predictor of 6 month death even when controlled for baseline BMI and eGFR. Conclusion Muscle area as measured at L3 vertebrae level on non-contrast enhanced CT is an strong predictor of 6 month mortality in elderly AAV patients receiving immunosuppressive therapy

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